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A neonate who was born at 24 weeks of gestational age died in few minutes after birth. In physical examination a distended abdomen, ambiguous genitalia and imperforated anus was seen. Autopsy revealed female gender, agenesis of left kidney and a large cystic mass containing 1.5 liter fluid with attachment to a small portion of the small intestine.
Pathology demonstrated a fibromuscular tissue that was covered by stratified squamous epithelium and one layer of cubeidal cells. Also unremarkable intestinal tissue was present.
The neonate’s mother was a 24 year old woman referred to the clinic because of severe abdominal distention, respiratory distress and palpitation. In ultrasound she revealed a single fetus with a large intra-abdominal mixed cystic mass measuring 108x65mm displacing normal organs and extending to the presacral area. Fat-fluid levels (fluid-fluid appearance) and severe poly-hydramnios was also observed. She underwent hystrotomy. She and her husband were first cousins. There was no family history of congenital anomalies or medication use during pregnancy.
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